We report a 59-year-old Japanese male with diabetic ulcer successfully treated with xenon ray irradiation. The patient, having diabetes mellitus and heart disease, presented with a 1-month history of an unhealed cutaneous ulcer on his left foot. No treatment with topical application was effective. Therefore, xenon ray irradiation was administered. After irradiation was performed with a total dose of 142.7 J/cm² over a treatment course of 10 weeks, the cutaneous ulcer improved. Xenon ray irradiation has a light source ranging from an ultra-violet wavelength to a near-infrared ray with a peak at 800-900 nm. Xenon ray treatment cutting off the ultra-violet wavelengh has been introduced for the control of pain and the rehabilitation of patients with motor disturbances. Near-infrared irradiation is used to promote wound healing. This result indicates that xenon ray irradiation can be valuable for the treatment of cutaneous ulcer in patients with diabetes mellitus.

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The case was a 43-year-old female with restiform objects on her head and along both temporal arteries and erythematous papules on her trunk. Histopathologically, significant infiltration of eosinophils was found as well as neutrophils and histiocytes from the intima to the media of the temporal arteries, indicating angitis. No giant cells were found. There were no systemic symptoms other than eruptions. Inflammatory indicators, including CRP, were not elevated. Diagnosis of this case was believed to be juvenile temporal arteritis with eosinophilia, which was advocated by Fujimoto et al., accompanied by dermatological symptoms.

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An 18-year-old woman with a 4-year history of asthma experienced joint pain 2 weeks prior to presentation at our hospital and developed purpura on the legs and feet in association with mild fever 5 days previous to presentation. Physical examination on admission revealed scattered pea-sized purpura, papules and hemorrhagic blisters on both legs and the dorsal aspect of the feet. She complained of fever and general fatigue. Laboratory findings revealed eosinophilia (4225 /μl) and high levels of serum IgE (2228 IU/ml), P-ANCA (227 EU) and rheumatoid factor (57.3 IU/ml). Skin biopsy was taken under the clinical diagnosis of vasculitis allergica cutis or allergic granulomatous angiitis (AGA). Histologically, necrotizing angiitis and dense perivascular infiltration of eosinophils with occasional granulomatous changes were observed in the entire dermis. The diagnosis of AGA was made and systemic administration of betamethasone (3 mg/day) was started. Although the skin lesions and general symptoms rapidly improved, eosinophilia was resistant to systemic steroid therapy. Additional administration of azathioprine (100 mg/day) for 2 months reduced the eosinophilia.

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We report a 38-year-old female patient with limited cutaneous systemic sclerosis. Within one month, there occurred rapid progression of edematous thickening in the bilateral forearm skin. We administered treatment with oral predonisolone at a dose of 20 mg/day. After two weeks, the modified Rodman total skin thickness score decreased from 15 to 9 points. In addition, we evaluated severity in various organs according to the classification of severity and the guidance of treatment of scleroderma published by the Ministry of Health, Labour and Welfare scleroderma investigation research group in 2004.

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A 54-year-old Japanese woman visited an ophthalmic clinic with headache and ophthalmalgia, and was diagnosed as having iritis of unknown etiology. One month later, she visited a dermatological clinic with a complaint of alopecia, and it was deemed to be related to Vogt-Koyanagi-Harada syndrome (VKH) by her history, and then she was referred to our clinic. At the first presentation, she had diffuse alopecia on the parietal region, and punched biopsy showed telogen hair follicules. Although poliosis and vitiligo were not observed, sunset glow fundii and sensorineural hearing loss were confirmed by an ophthalmologist and an otologist. Therefore, she was diagnosed as having VKH. VKH should be considered for the differential diagnosis of alopecia as well as poliosis and vitiligo.

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A 74-year-old man noticed a macule on the right side of his back in July 2001. The macule gradually increased in size thereafter. After visiting a clinic, he was referred to us. The macule was brown-reddish, slightly elevated, with a red nodule. We diagnosed the lesion as a poroma (Smith-Coburn type) based on histological examination of the nodule. We then excised the entire lesion. Histopathologically the macular lesion had histological features of seborrheic keratosis (clonal type) associated with poroma (Smith-Coburn type). This case is considered very rare.

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A 42-year-old woman had noticed a painful and bleeding tumor at the umbilicus over a period of 6 months. Physical examination revealed a smooth-surfaced, hard, brown tumor of 1 cm in diameter. Biopsy specimen showed endometrial composed of one layer of columnar epithelium. The lesion was diagnosed as umbilical endometriosis. Transvaginal ultrasound and abdominal MRI examination revealed bilateral ovarian endometrial cysts. A large endometrial cyst on the left ovary and the umbilical tumor were removed surgically. This case suggests that when we see a patient with umbilical endometriosis, we should also consider the possibility of intrapelvic endometriosis, such as ovarian endometriosis, and consult a gynecologist.

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A 28-year-old woman was bitten by an adder in the 34th week of her pregnancy, resulting in delivery of a baby girl by caesarean section on the following day due to potentially fatal asphyxia. Although the mother went into acute renal and respiratory failure and disturbance of consciousness due to rabdomyolysis and the baby developed asphyxia neonatorum and respiratory distress syndrome, both survived through the combined treatment of antitoxic serum and effective supportive care.

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The patient was a 55-year-old man with chronic inflammatory demyelinating polyradiculoneuropathy, for which high-dose immunoglobulin therapy was administered for 5 days. On the fifth day of therapy, he developed small blisters on the fingers of both hands, then on both soles. He was referred to our department. At initial examination, numerous slightly itchy small blisters and serous papules were observed on the fingers of both hands, both palms, and both soles. Histopathological examination showed spongiosis and vesiculation in the epidermis and perivascular lymphocytic infiltration in the superficial corium. Dyshidrotic eczema was diagnosed from the clinical and histopathological findings. We report and discuss dyshidrotic eczema developing after high-dose immunoglobulin therapy for demyelinating neuropathy.

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We report a case of post-transplantation lymphoma (intra-cranial EBV-related malignant lymphoma) who developed toxic epidermal necrolysis (TEN) during concomitant phenobarbital administration and radiotherapy. The erythema exsudativum multiforme-like eruption first appeared on the site of radiation and extended to approximately 35% of the body surface. After stopping radiation therapy and all medications, including immunosuppressant, anticonvulsant, and diuretic drugs, treatment was successfully administered by systemic corticosteroids including semi-pulse therapy (500 mg of methylpredonisolone sodium succinate for 3 days).

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A 48-year-old Japanese lady was examined in 1989. She was apparently healthy but showed numerous numbers of small petechiae-like angiokeratoma on her lower torso to upper thighs, axillae and beneath the breasts. Electron microscopic examination of the skin revealed largely dilated electron lucent lysosomes with fuzzy filamentous materials in vascular endothelial cells, fibroblasts, eccrine sweat gland cells and others. Urinary examination revealed unusual glycopeptides with GalNAc-Ser/Thr moieties. This disease was reported as a novel lysosomal storage disease with angiokeratoma corporis diffusum, crowned Kanzaki disease (MIM#104170). Soon, this disease was found to be caused by a deficit of α-N-acetylgalactosaminidase (α-NAGA, 4. 3. 2. 49) activity and a point mutation was found in the gene (R329W) encoding the enzyme. Another patient, 47-year-old Japanese woman, was found, and she also was apparently healthy, but had less angiokeratoma as compared to the first one. The gene mutation was found and the resultant mutant enzyme was R329Q. She excreted less amount of GalNAc-Ser in urine as compared to the first patient. These phenotypical differences between case 1 and 2 were estimated to be caused by the differences in the three-dimensional structures in mutated α-NAGAs (R329W v. s. R329Q). Schindler disease also shows α-NAGA deficiency but shows very severe central nervous symptoms before the age of one. Electron microscopically electron-dence material deposited in lysosomes in Schindler disease. Electron-dense material means that the substance are probably lipid- or lipoprotein-containg materials. This quiet contrasts to the findings in Kanzaki disease. These evidences suggest that Kanzaki disease is caused by a pure α-NAGA deficiency but Schindler disease is probably caused by together α-NAGA deficiency with some other factors.

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Significance of the drug patch test (PT) was evaluated in 1650 reported cases of drug eruption. Positivity rates were analyzed with regard to the types of exanthema, categories of drugs and individual drugs. The positive PT rate in the total of 1650 cases was 55.4%. Higher positive rates were observed in the following types of drug eruptions with epidermal changes induced by sensitized T lymphocytes: eczematous eruption, erythema multiforme, pityriasis rosea-like eruption, maculopapular rash, erythroderma and drug-induced hypersensitivity syndrome. The positivity rate in fixed drug eruption was 100% when the drugs were applied to the involved skin. In contrast, the rate was low in the types of scratch dermatitis, drug-induced SLE and pemphigus, and purpura. Additionally, in the analysis according to individual drugs, it was clarified that there were two drug groups of 100% and 0% at the positivity rate. The positive PT rate was 100% for clindamycin, iomeprol, beraprost sodium, mexiletine and diflunisal, but there was no positive PT for interferon-α, tosufloxacin tosilate, oxatomide, diaphenylsulfone, cinnarizine and methazolamide. It seems that these findings frequently serve as a reference in performing PT or interpreting the results of PT.

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We evaluated the effect of olopatadine hydrochloride (allelock^®) on sleep loss induced from itch, which is a major symptom of atopic dermatitis, by using pruritic scores and actigraphy. Nocturnal movements of 3 patients with atopic dermatitis were measured for 7 to 10 consecutive nights by actigraphy. These patients had taken allelock^® for at least 3 consecutive days while undergoing actigraphy. Pruritic scores were described before patients went to bed. We found a significant correlation between the mean activity score and the pruritic score and between the mean score during active periods and the pruritic score. Two of 3 cases recognized the efficacy of oral allelock^®. The pruritic scores decreased in these 2 cases, and the number of nocturnal movements decreased in one case. On the other hand, neither the pruritic score nor the number of nocturnal movements decreased in the case that did not recognize efficacy of the drug. These results indicate that measurement of nocturnal movements using actigraphy may be an appropriate method to evaluate nocturnal itch.

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The efficacy and safety of bepotastine besilate (Talion^®) in the treatment of chronic urticaria were evaluated in 88 adult patients with chronic urticaria. Patients were openly treated with 20 mg/day oral bepotastine besilate for 14 days. Clinical evaluations were performed every 7 days, and patients were asked to keep a diary, recording their pruritus scores. Symptoms improved in 77.1% of the patients after two weeks and pruritus scores were reduced significantly within 24 h after oral administration in 69.6% of patients. It appears that bepotastine besilate can be ranked as a drug of first choice for the treatment of chronic urticaria, because it rapidly relieved itch and the incidence of drowsiness was relatively low.

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Although many studies have shown that active vitamin D₃ ointment is effective for the treatment of psoriasis, to our knowledge there are no studies that directly compare topical steroid monotherapy and combination therapy consisting of topical steroid and active vitamin D₃. The aim of our study was to compare the efficacy, safety, and compliance between diflucortolone valerate ointment monotherapy and combination therapy with weekday diflucortolone valerate and weekend calcipotriol ointment. Psoriasis patients were treated twice daily for 6 weeks with diflucortolone valerate (20 patients) or diflucortolone valerate/calcipotriol (24 patients). Both treatments significantly improved the psoriatic skin with similar clinical efficacy. No side effects were observed during the treatment. Questionnaire administered to patients after the study revealed that compliance and satisfaction with the two treatments were similar. Thus, we concluded that diflucortolone valerate/calcipotriol combination therapy was similarly potent as diflucortolone valerate ointment monotherapy and was a good modality as an alternative to such monotherapy for the treatment of psoriasis.

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We administered combination therapy consisting of maxacalcitol ointment with bath-PUVA therapy in 11 patients with psoriasis vulgaris and studied the effect of the therapy and the degree of pigmentation caused by this therapy. Patients were hospitalized for 4 weeks and were treated with topical maxacalcitol ointment twice a day in combination of bath-PUVA therapy 3 times a week. Evaluated were the severity of skin eruptions including pigmentation and PASI scores and the degree of general improvement. Scores for erythema, indurated lesions and scales and PASI scores decreased significantly after two weeks, and then further decreased after a month. All patients had improved significantly after a month. On the other hand, although the degree of pigmentation had increased significantly after 2 and 4 weeks, that at 6 months after discharge was almost the same as before therapy. Therefore, we thought that the pigmentation was almost all postinflammatory pigmentation, which had been accelerated by the combination therapy.

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